Article
The β1-subunit of Na(v)1.5 cardiac sodium channel is required for a dominant negative effect through α-α interaction.
PloS one - 1 Jan 2012
Mercier Aurélie, Clément Romain, Harnois Thomas, Bourmeyster Nicolas, Faivre Jean-François, Findlay Ian, Chahine Mohamed, Bois Patrick, Chatelier Aurélien
Abstract excerpt
Brugada syndrome (BrS) is an inherited autosomal dominant cardiac channelopathy. Several mutations on the cardiac sodium channel Na(v)1.5 which are responsible for BrS lead to misfolded proteins that do not traffic properly to the plasma membrane. In order to mimic patient heterozygosity, a traff...
Topics
- Brugada Syndrome
- DNA, Complementary
- Electrophysiology
- Genes, Dominant
- Green Fluorescent Proteins
- HEK293 Cells
- Heterozygote
- Humans
- Immunoblotting
- Immunoprecipitation
- Microscopy, Fluorescence
- Mutation
- NAV1.5 Voltage-Gated Sodium Channel
- Patch-Clamp Techniques